Characterized by a triad of eczema, recurrent infections along with elevated IgE levels, hyper-IgE syndromes refer to a heterogeneous group of monogenic inborn immune disorders with either autosomal dominant (AD) or autosomal recessive (AR) inheritance, namely AD-HIES or AR-HIES, respectively [8,9]. Here, IGHE is linked to Eczematoid dermatitis.